Immunology Flashcards

1
Q

what do primary immunodeficiencies often present with?

A

recurrent respiratory tract infections

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2
Q

what do primary antibody deficiencies often present with?

A

sinusitis

otitis media

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3
Q

four examples of primary antibody deficiencies

A
  • selective IgA deficiency
  • common variable immunodeficiency (CVID)
  • specific antibody deficiency (SAD)
  • X-linked agammaglobulinemia (XLA)
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4
Q

what do complement disorders often present with

A

laryngeal angioedema

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5
Q

three stages where development of neutrophils can go wrong

A
  1. defects in development
  2. defects in trans-endothelial migration
  3. defects in neutrophil killing
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6
Q

example of a condition that results in defects in neutrophil development

A

severe congenital neutropenia (Kostmann syndrome)

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7
Q

how can severe congenital neutropenia (Kostmann syndrome) be detected

A

accumulation of precursor cells in the bone marrow

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8
Q

example of a condition that results in defects in neutrophil transendothelial migration

A

leukocyte adhesion deficiency

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9
Q

example of a condition that result in defects in neutrophil killing

A

chronic granulomatous disease (CGD)

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10
Q

what does CGD cause?

A

inability to generate ROS/RNS

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11
Q

management for conditions with defects in neutrophil production

A
  • immunoglobulin replacement therapy
  • management of infection
  • stem cell transplantation
  • gene therapy
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12
Q

therapy for CGD

A

IFN-gamma therapy

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13
Q

which bacterial species can hide within immune cells?

A

mycobacteria species

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14
Q

what is the defence network against mycobacteria species called?

A

IL-12 IFN-gamma network

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15
Q

describe the IL-12 IFN-gamma network

A
  • infected macrophages produce IL-12
  • this stimulates NK cells and TH1 to secrete IFN-gamma
  • this stimulates macrophages to activate NADPH oxidase complex
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16
Q

five stages where adaptive immune system production can go wrong?

A
  1. defects in haemopoietic stem cells
  2. defects in lymphoid precursor cells
  3. defects in thymocyte development
  4. defects in B cell development
  5. defects in B cell effector functions
17
Q

example of a condition which results in defects in haemopoietic stem cells

A

reticular dysgenesis (fatal unless HSC transplantation)

18
Q

example of a condition which results in defects in lymphoid precursor cells

A

severe combined immunodeficiencies (SCID)- both T and B cells are not produced. commonest form is X-linked SCID

19
Q

example of a condition that results in defects in thymocyte development

A

DiGeorge syndrome

20
Q

example of a condition that results in defects in B cell development

A

X-linked agammaglobulinaemia (XLA)

21
Q

two conditions that result in defects in B cell effector functions

A

hyper IgM syndrome

selective IgA deficiency

22
Q

define hypersensitivity reaction

A

abnormal immune response that results in bystander damage to self

23
Q

how are hypersensitivity reactions divided?

A

Gel and Coomb’s classification

24
Q

type 1 hypersensitivity reaction

A

immediate hypersensitivity where IgE antibody response to an external antigen.

25
Q

for type 1 hypersensitivity what happens when an atopic individual comes into contact with an allergen

A

TH2 response (as well as TH1) which produces mast cells and eosinophils which express Fc receptors for the antibody and when the allergen binds it disrupts the IgE membrane releasing histamine

26
Q

example of type 1 hypersensitivity reaction

A

allergic reaction

27
Q

management of type 1 hypersensitivity reaction

A

avoid allergen
block mast cells
anti-inflammatory agents
immunotherapy

28
Q

what is a type 2 hypersensitivity reaction?

A

direct cell effects: IgM or IgG antibodies attack the cell surface (autoantibodies)

29
Q

example of a type 2 hypersensitivity reaction

A

Goodpasture’s syndrome

30
Q

goodpastures syndrome

A

IgM or IgG attack the basement membrane of cells in the lungs and kidneys

31
Q

management of good pastures syndrome

A
  • corticosteroids
  • plasmapheresis
  • stop smoking
32
Q

type 3 hypersensitivity reaction

A

immune complex mediated: immune complexes are deposited in blood vessel walls where their presence initiates inflammatory cells (neutrophil releases enzyme that damage the basement membrane)

33
Q

example of a type 3 hypersensitivity reaction

A

hypersensitivity pneumonitis

34
Q

management of pneumonitis

A

avoidance
corticosteroids
immunosuppression

35
Q

type 4 hypersensitivity reaction

A

delayed type hypersensitivity

36
Q

examples of type 4

A

TB

sarcoidosis

37
Q

what happens in type 4

A

inhalation of antigen results it activation of immune cells leading to granuloma formation which causes tissue damage and fibrosis

38
Q

management of type 4

A

NSAIDS

corticosteroids